HUS
MCID: HML001
MIFTS: 53

Hemolytic-Uremic Syndrome (HUS)

Categories: Blood diseases, Immune diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Hemolytic-Uremic Syndrome

MalaCards integrated aliases for Hemolytic-Uremic Syndrome:

Name: Hemolytic-Uremic Syndrome 12 74 29 54 6 43 15 71
Hemolytic Uremic Syndrome 12 52 58
Haemolytic-Uraemic Syndrome 12 32
Hus 52 58
Acute Renal Failure, Thrombocytopenia, and Microangiopathic Hemolytic Anemia Associated with Distorted Erythrocytes 52

Classifications:

Orphanet: 58  
Rare renal diseases
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:12554
ICD9CM 34 283.11
MeSH 43 D006463
NCIt 49 C75545
SNOMED-CT 67 111407006
ICD10 32 D59.3
Orphanet 58 ORPHA544458
UMLS 71 C0019061

Summaries for Hemolytic-Uremic Syndrome

NIH Rare Diseases : 52 Hemolytic uremic syndrome (HUS) is a disorder that usually occurs when an E. coli bacterial infection in the digestive system produces toxic substances that destroy red blood cells. Symptoms include vomiting and diarrhea, fever, lethargy , and weakness. In severe cases it can lead to kidney failure or death. While this condition is most common in children, it often has a more complicated presentation in adults. Treatment may include dialysis , corticosteroids , transfusions of packed red blood cells and plasmapheresis . Hemolytic uremic syndrome should be distinguished from atypical hemolytic uremic syndrome (aHUS). The two conditions have different causes and different signs and symptoms.

MalaCards based summary : Hemolytic-Uremic Syndrome, also known as hemolytic uremic syndrome, is related to atypical hemolytic uremic syndrome with complement gene abnormality and genetic atypical hemolytic-uremic syndrome. An important gene associated with Hemolytic-Uremic Syndrome is DGKE (Diacylglycerol Kinase Epsilon), and among its related pathways/superpathways are Innate Immune System and Immune response Lectin induced complement pathway. The drugs Tacrolimus and Triamcinolone have been mentioned in the context of this disorder. Affiliated tissues include kidney, endothelial and bone, and related phenotypes are hematopoietic system and homeostasis/metabolism

Disease Ontology : 12 A kidney disease that is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs.

Wikipedia : 74 Hemolytic-uremic syndrome (HUS) is a group of blood disorders characterized by low red blood cells,... more...

Related Diseases for Hemolytic-Uremic Syndrome

Diseases in the Hemolytic-Uremic Syndrome family:

D-Plus Hemolytic Uremic Syndrome

Diseases related to Hemolytic-Uremic Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 839)
# Related Disease Score Top Affiliating Genes
1 atypical hemolytic uremic syndrome with complement gene abnormality 34.0 THBD CFI CFH CFB CD46 C3
2 genetic atypical hemolytic-uremic syndrome 33.5 THBD DGKE CFI CFHR4 CFHR3 CFHR1
3 complement factor h deficiency 33.4 CFI CFHR5 CFHR1 CFH CD46
4 acute kidney failure 32.3 HP CFH ADAMTS13
5 thrombotic microangiopathy 32.0 VWF HP CFH CD46 ADAMTS13
6 purpura 31.8 VWF THBD F3 CD40LG C3 ADAMTS13
7 hemolytic uremic syndrome, atypical 1 31.6 VWF THBD MMACHC HP F3 DGKE
8 disseminated intravascular coagulation 31.5 VWF THBD F3 ADAMTS13
9 anuria 31.5 HP F3 CD40LG ADAMTS13
10 end stage renal disease 31.5 THBD CFI CFHR5 CFHR3 CFHR1 CFH
11 kidney cortex necrosis 31.4 MMACHC ADAMTS13
12 severe pre-eclampsia 31.4 CFH CFB C3
13 cfhr5 deficiency 31.3 CFHR5 CFHR1
14 kidney disease 31.2 THBD HP F3 CFHR5 CFH C3
15 thrombotic thrombocytopenic purpura, hereditary 31.2 HP CFH ADAMTS13
16 diarrhea 31.1 THBD CFI CFH CFB CD55 CD46
17 glomerulonephritis 31.1 CFI CFHR5 CFH CD55 CD46 CD40LG
18 malignant hypertension 31.0 VWF THBD CFHR2 CFH CD46 ADAMTS13
19 methylmalonic acidemia 31.0 MMACHC DGKE CFHR2 ADAMTS13
20 chronic kidney disease 31.0 THBD HP CFI CFHR5 CFHR1 CFH
21 vasculitis 31.0 VWF THBD CD40LG C3
22 neisseria meningitidis infection 31.0 CD46 C3
23 raynaud phenomenon 30.9 VWF THBD
24 thrombotic thrombocytopenic purpura 30.9 VWF THBD HP F3 CFI CFH
25 macular degeneration, age-related, 6 30.9 CFH CFB
26 proliferative glomerulonephritis 30.8 CFHR2 CFH CD55 C3
27 macular degeneration, age-related, 14 30.8 CFB C2
28 thrombophilia 30.8 VWF THBD F3 CD40LG
29 acute poststreptococcal glomerulonephritis 30.8 CFHR5 CFHR2 CD40LG C3
30 bilirubin metabolic disorder 30.8 HP F3 CD40LG ADAMTS13
31 membranoproliferative glomerulonephritis 30.8 DGKE CFHR5 CFHR2 CFH CFB CD46
32 hemoglobinuria 30.8 THBD HP CFI CD55 C3
33 hypertensive retinopathy 30.8 VWF ADAMTS13
34 complement factor i deficiency 30.7 CFI C3
35 iga glomerulonephritis 30.6 CFHR5 CFHR3 CFHR2 CFHR1 CFH CD40LG
36 enterocolitis 30.6 THBD CFI CFHR5 CFHR3 CFHR1 CFH
37 meningitis 30.6 CFI CD55 CD46 CD40LG C3
38 catastrophic antiphospholipid syndrome 30.6 F3 CFH CD40LG ADAMTS13
39 paroxysmal nocturnal hemoglobinuria 30.6 THBD HP CFI CD55 C3
40 evans' syndrome 30.6 HP CD40LG ADAMTS13
41 dense deposit disease 30.5 DGKE CFHR5 CFHR2 CFH CFB C3
42 collagen disease 30.5 VWF THBD CD40LG
43 von willebrand's disease 30.5 VWF F3 ADAMTS13
44 complement component 5 deficiency 30.5 CFHR2 CD55 CD46 C3
45 henoch-schoenlein purpura 30.5 CFHR2 CD40LG C3
46 immune-complex glomerulonephritis 30.5 CFI CFHR2 CD40LG
47 heparin-induced thrombocytopenia 30.4 F3 CD40LG
48 homocysteinemia 30.4 VWF THBD MMACHC
49 c3 glomerulopathy 30.4 DGKE CFI CFHR5 CFHR3 CFHR2 CFHR1
50 anemia, autoimmune hemolytic 30.3 HP CD55 CD40LG C3

Comorbidity relations with Hemolytic-Uremic Syndrome via Phenotypic Disease Network (PDN):


Acute Kidney Failure Deficiency Anemia
Heart Disease

Graphical network of the top 20 diseases related to Hemolytic-Uremic Syndrome:



Diseases related to Hemolytic-Uremic Syndrome

Symptoms & Phenotypes for Hemolytic-Uremic Syndrome

MGI Mouse Phenotypes related to Hemolytic-Uremic Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10 ADAMTS13 C3 CD40LG CD55 CFB CFH
2 homeostasis/metabolism MP:0005376 9.97 ADAMTS13 C3 CD40LG CD55 CFB CFH
3 immune system MP:0005387 9.7 ADAMTS13 C3 CD40LG CD55 CFB CFH
4 renal/urinary system MP:0005367 9.28 C3 CD40LG CD55 CFB CFH CFI

Drugs & Therapeutics for Hemolytic-Uremic Syndrome

Drugs for Hemolytic-Uremic Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 67)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tacrolimus Approved, Investigational Phase 4 104987-11-3 445643 439492 6473866
2
Triamcinolone Approved, Vet_approved Phase 4 124-94-7 31307
3
Mycophenolic acid Approved Phase 4 24280-93-1 446541
4
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
5
Abatacept Approved Phase 4 332348-12-6 10237
6
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
7
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
8 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
9
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
10
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
11 Immunologic Factors Phase 4
12 Immunosuppressive Agents Phase 4
13 Antirheumatic Agents Phase 4
14 Hematinics Phase 4
15 Epoetin alfa Phase 4 113427-24-0
16 Antitubercular Agents Phase 4
17 Anti-Infective Agents Phase 4
18 Triamcinolone diacetate Phase 4
19 Anti-Bacterial Agents Phase 4
20 triamcinolone acetonide Phase 4
21 Triamcinolone hexacetonide Phase 4
22 Antibiotics, Antitubercular Phase 4
23 Calcineurin Inhibitors Phase 4
24 Methylprednisolone Acetate Phase 4
25
Ravulizumab Approved, Investigational Phase 3 1803171-55-2
26 Pharmaceutical Solutions Phase 2, Phase 3
27 Immunoglobulins, Intravenous Phase 2, Phase 3
28 Shiga Toxins Phase 3
29
Promethazine Approved, Investigational Phase 2 60-87-7 4927
30
Acetaminophen Approved Phase 2 103-90-2 1983
31
Diphenhydramine Approved, Investigational Phase 2 58-73-1, 147-24-0 3100
32
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
33
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
34 Antibodies Phase 2
35 Antibodies, Monoclonal Phase 2
36 Immunoglobulins Phase 2
37 Liver Extracts Phase 2
38 Complement System Proteins Phase 2
39 Androgen Receptor Antagonists Phase 2
40 Immunotoxin HA22 Phase 2
41 Immunotoxins Phase 2
42
Aspirin Approved, Vet_approved Phase 1 50-78-2 2244
43
rituximab Approved Phase 1 174722-31-7 10201696
44 Angiotensin-Converting Enzyme Inhibitors Phase 1
45 Vaccines Phase 1
46 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 1
47 Antineoplastic Agents, Immunological Phase 1
48
Metoclopramide Approved, Investigational 364-62-5 4168
49
Methotrexate Approved 59-05-2, 1959-05-2 126941
50
leucovorin Approved 58-05-9 6006 143

Interventional clinical trials:

(show top 50) (show all 64)
# Name Status NCT ID Phase Drugs
1 Multicentric, Prospective Open-label Study Assessing an Algorithm-based Strategy of Eculizumab Discontinuation in Children and Adults With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT02574403 Phase 4 eculizumab
2 Effect of Erythropoietin on Red Blood Cell Requirement in Children With Hemolytic Uremic Syndrome: a Randomized Controlled Trial Recruiting NCT03776851 Phase 4 erythropoietin
3 Randomized Conversion Of Epstein-Barr Virus (EBV)+ Kidney Transplant Recipients Of Living Or Standard Criteria Donors At Three Months Post Transplantation To Belatacept With MPA Or Belatacept With Low-Dose Tacrolimus (50% Of Dose) Compared To Patients Remaining On Center Specific Standard Therapy Of Tacrolimus And MPA Recruiting NCT02213068 Phase 4 belatacept;Tacrolimus;MPA
4 An Open-Label, Multi-Center Trial of Eculizumab in Patients With Shiga-Toxin Producing Escherichia Coli Hemolytic-Uremic Syndrome (STEC-HUS) Completed NCT01410916 Phase 2, Phase 3 Eculizumab (Soliris®)
5 Early Treatment With the Monoclonal C5 Antibody Eculizumab in Pediatric Patients Affected by Shiga-toxin Related Hemolytic and Uremic Syndrome: A Phase III Prospective Randomized Controlled Therapeutic Trial Versus Placebo Completed NCT02205541 Phase 3 Eculizumab;Placebo
6 A Phase 3 Study to Evaluate the Safety and Efficacy of OMS721 for the Treatment of Atypical Hemolytic Uremic Syndrome (aHUS) in Adults and Adolescents Recruiting NCT03205995 Phase 3
7 CONSERVE: rVA576 (Coversin) Long Term Safety and Efficacy Surveillance Study Recruiting NCT03829449 Phase 3 rVA576 (Coversin)
8 A Double-blind, Placebo-controlled, Adaptive, Phase 2/3 Study to Evaluate the Pharmacokinetics, Safety, and Efficacy of INM004 in Pediatric Patients With Shiga Toxin-positive Bloody Diarrhea for Prevention of Hemolytic Uremic Syndrome Recruiting NCT04132375 Phase 2, Phase 3 INM004;Placebo
9 Improvements Through the Use of a Rapid Multiplex PCR Enteric Pathogen Detection Kit in Children With Hematochezia Recruiting NCT03362970 Phase 3
10 A Phase 3, Open-Label, Multicenter Study of ALXN1210 in Children and Adolescents With Atypical Hemolytic Uremic Syndrome (aHUS) Active, not recruiting NCT03131219 Phase 3
11 Single Arm Study of ALXN1210 in Complement Inhibitor Treatment-naïve Adult and Adolescent Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Active, not recruiting NCT02949128 Phase 3
12 Phase III Randomized Study of SYNSORB Pk in Children With E. Coli-Associated Terminated NCT00004465 Phase 3 SYNSORB Pk;Placebo
13 The Plasma Large-Volume Exchange Randomized Controlled Trial (PLEX-RCT) Withdrawn NCT01433003 Phase 3
14 A Phase II Study Evaluating the Efficacy of Rituximab in the Management of Patients With Relapsed/Refractory Thrombotic Thrombocytopenic Purpura (TTP) - Hemolytic Uremic Syndrome (HUS) Unknown status NCT00531089 Phase 2 Rituximab
15 The Safety and Efficacy of Eculizumab in Japanese Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT01757431 Phase 2 Eculizumab
16 An Open-Label, Multi-Center Clinical Trial of Eculizumab in Pediatric Patients With Atypical Hemolytic-Uremic Syndrome Completed NCT01193348 Phase 2 Eculizumab
17 An Open-label, Multi-center Clinical Trial of Eculizumab in Adult Patients With Atypical Hemolytic-uremic Syndrome Completed NCT01194973 Phase 2 Eculizumab
18 An Open-Label, Multi-Center Controlled Clinical Trial of Eculizumab in Adolescent Patients With Plasma Therapy-Resistant Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT00844844 Phase 2 Eculizumab
19 An Open-Label, Multi-Center Controlled Clinical Trial of Eculizumab in Adult Patients With Plasma Therapy-Resistant Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT00844545 Phase 2 Eculizumab
20 An Open-Label, Multi-Center Controlled Clinical Trial Of Eculizumab in Adolescent Patients With Plasma Therapy-Sensitive Atypical Hemolytic Uremic Syndrome (AHUS) Completed NCT00844428 Phase 2 eculizumab
21 An Open-label, Multi-center Controlled Clinical Trial of Eculizumab in Adult Patients With Plasma Therapy-sensitive Atypical Hemolytic Uremic Syndrome (AHUS) Completed NCT00838513 Phase 2 eculizumab
22 A Phase II Study of Chimeric Monoclonal Antibodies to Shiga Toxins 1 (cαStx1) and 2 (cαStx2) Administered Concomitantly to Children With Shiga Toxin-Producing Bacterial (STPB) Infection and Bloody Diarrhea (SHIGATEC Trial) Completed NCT01252199 Phase 2 cαStx1/cαStx2;Placebo
23 Early Intervention With Eculizumab to Treat Thrombotic Microangiopathy/Atypical Hemolytic Uremic Syndrome (TMA/aHUS)-Associated Multiple Organ Dysfunction Syndrome (MODS) in Hematopoietic Stem Cell Transplant (HCT) Recipients Recruiting NCT03518203 Phase 2 Eculizumab
24 A Phase 2, Uncontrolled, Three-Stage, Dose-Escalation Cohort Study to Evaluate the Safety, Pharmacokinetics, Pharmacodynamics, Immunogenicity, and Clinical Activity of OMS721 in Adults With Thrombotic Microangiopathies Recruiting NCT02222545 Phase 2
25 A Phase II Clinical Trial of Anti-Tac(Fv)-PE38 (LMB-2) Immunotoxin for CD25 Positive Hairy Cell Leukemia Active, not recruiting NCT00321555 Phase 2 Anti-Tac(Fv)-PE38 (LMB-2) Immunotoxin
26 A Phase II, Randomized, Double-blind, Placebo-controlled Trial to Evaluate the Safety and Efficacy of Cemdisiran (ALN-CC5) Following Withdrawal of Chronic Eculizumab Therapy in Patients With Atypical HUS at High Risk of Recurrence Not yet recruiting NCT03999840 Phase 2 cemdisiran;Placebos
27 An Open-label Phase 2 Study to Assess the Effect of C5aR Antagonist Therapy by CCX168 Oral Administration on ex Vivo Thrombus Formation and Disease Activity in ESRD Patients With Atypical Hemolytic Uremic Syndrome Terminated NCT02464891 Phase 2 CCX168
28 A Phase II Study of the Anti-CD22 Recombinant Immunotoxin Moxetumomab Pasudotox (CAT-8015, HA22) in Children With B-lineage Acute Lymphoblastic Leukemia and Minimal Residual Disease Prior to Allogeneic Hematopoietic Stem Cell Transplantation Terminated NCT02338050 Phase 2
29 Retreatment Protocol for BL22 Immunotherapy in Relapsed or Refractory Hairy Cell Leukemia Terminated NCT00924040 Phase 2 BL22 (CAT-3888)
30 A Phase 2, Open Label, Multicenter Study of ALN-CC5 Administered Subcutaneously in Adult Patients With Atypical Hemolytic Uremic Syndrome Withdrawn NCT03303313 Phase 2 Cemdisiran
31 A Phase I Study to Evaluate the Safety, Tolerance and Pharmacokinetics of the Anti-Shiga Toxin Hyperimmune Equine Immunoglobulin F(ab')2 Fragment (INM004) in Healthy Volunteers Completed NCT03388216 Phase 1
32 Eculizumab Therapy for Dense Deposit Disease and C3 Nephropathy Completed NCT01221181 Phase 1 Eculizumab
33 A Phase I Study of Moxetumomab Pasudotox-tdfk (Lumoxiti (TM)) and Rituximab (Rituxan (R)) for Relapsed Hairy Cell Leukemia Recruiting NCT03805932 Phase 1 Moxetumomab Pasudotox-tdfk
34 Inpatient Volume Expansion in Children With Shiga Toxin-Producing Escherichia Coli (STEC) Infection to Prevent Hemolytic Uremic Syndrome (HUS) Not yet recruiting NCT03275792 Phase 1 D5-0.9%NS;Routine home oral rehydration
35 Eculizumab in HELLP Syndrome Not yet recruiting NCT04103489 Phase 1 Eculizumab
36 Complement Activation During Hemodialysis in Atypical Hemolytic Uraemic Syndrome as Underlying Kidney Disease. Unknown status NCT00930423
37 Study of 'Vascular Competence' Profile and Endothelial Activation in the Hemolytic Uremic Syndrome in Children and Adults Unknown status NCT02904863
38 A Research Study to Describe the Safety and Efficacy of Eculizumab in Japanese Patients With Atypical Hemolytic Uremic Syndrome (aHUS). An Assessment of Two Case Studies Completed NCT01755429
39 A Retrospective, Observational, Non-interventional Trial to Assess Eculizumab Treatment Effect in Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT01770951
40 Haemolytic Uraemic Syndrome in Childhood: Clinical, Cognitive and Psychological Aspects Completed NCT01666548
41 Outbreak of Hemolytic Uremic Syndrome Linked to Escherichia Coli of Serotype O104:H4 in Bordeaux Urban Area, June 2011: Evaluation of Diagnostic, Prognostic and Pathophysiological Data Completed NCT01406288
42 Repetitive Intestinal Lavage Using Polyethylene Glycol Solution in Patients With EHEC O104:H4 Infection During the German 2011 Outbreak for Prevention of Severe Thrombocytopenia With Subsequently Following Therapeutic Plasmapheresis Completed NCT01561248 polyethylene glycol solution for daily bowel lavage.
43 The Role of Endothelium Dysfunction in Progression of CKD (Chronic Kidney Disease) After AKI (Acute Kidney Injury) Completed NCT00358306
44 Association Between the Quantitative Assessment of Schistocytes in Peripheral Blood Smear and Prognosis of Patient Initially Diagnosed as HELLP Syndrome. Completed NCT03246542
45 Assessment of Immature Platelet Fraction in Pregnancy-Associated Thrombotic Microangiopathy Completed NCT03232359
46 Safety of Tacrolimus And Methotrexate (MTX) Versus Tacrolimus And Mycophenolate Mofetil (MMF) As Graft Versus Host Disease Prophylaxis In Allogeneic Hematopoietic Cell Transplants (HCT) Completed NCT00360685 Tac+MTX;TAC + MMF
47 A Observational Study to Determine the Prevalence of Pregnancy-related Thrombotic Thrombocytopenic Purpura and Atypical Haemolytic Uraemic Syndrome in Women Affected by Specific Obstetric Complications Recruiting NCT03605511
48 An Observational, Non-Interventional, Multi-Center, Multi-National Study of Patients With Atypical Hemolytic-Uremic Syndrome (aHUS Registry) Recruiting NCT01522183
49 US Post-Marketing Retrospective Observational Safety Study of Moxetumomab Pasudotox-tdfk (LUMOXITI)(TM) Recruiting NCT04125290
50 Coordination of Rare Diseases at Sanford Recruiting NCT01793168

Search NIH Clinical Center for Hemolytic-Uremic Syndrome

Cochrane evidence based reviews: hemolytic-uremic syndrome

Genetic Tests for Hemolytic-Uremic Syndrome

Genetic tests related to Hemolytic-Uremic Syndrome:

# Genetic test Affiliating Genes
1 Hemolytic-Uremic Syndrome 29

Anatomical Context for Hemolytic-Uremic Syndrome

MalaCards organs/tissues related to Hemolytic-Uremic Syndrome:

40
Kidney, Endothelial, Bone, Colon, Bone Marrow, Liver, Heart

Publications for Hemolytic-Uremic Syndrome

Articles related to Hemolytic-Uremic Syndrome:

(show top 50) (show all 5767)
# Title Authors PMID Year
1
Rifaximin does not induce toxin production or phage-mediated lysis of Shiga toxin-producing Escherichia coli. 61 54
17526759 2007
2
Determination of complement factor H functional polymorphisms (V62I, Y402H, and E936D) using sequence-specific primer PCR and restriction fragment length polymorphisms. 54 61
17022693 2006
3
Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS 13 deficiency? 61 54
16411392 2005
4
Shear stress and von Willebrand factor in health and disease. 54 61
14631548 2003
5
Assays of von Willebrand factor-cleaving protease: a test for diagnosis of familial and acquired thrombotic thrombocytopenic purpura. 61 54
11992240 2002
6
Function of von Willebrand factor in children with diarrhea-associated hemolytic-uremic syndrome (D+ HUS). 54 61
11446662 2001
7
Hemolytic-uremic syndrome and complement factor H deficiency: clinical aspects. 61 54
11446651 2001
8
Problems with platelet counting in thrombocytopenia. A rapid manual method to measure low platelet counts. 61 54
11446657 2001
9
Recurrent and de novo renal disease after kidney transplantation with or without cyclosporine A. 54 61
2024653 1991
10
The role of von Willebrand factor (vWF) in thrombotic thrombocytopenic purpura (TTP) and the hemolytic-uremic syndrome (HUS). 54 61
2191307 1990
11
A novel compound heterozygous mutation in DGKE in a Chinese patient causes atypical hemolytic uremic syndrome. 61
32091318 2020
12
Production of egg yolk antibody (IgY) against shiga-like toxin (stx) and evaluation of its prophylaxis potency in mice. 61
32320733 2020
13
Molecular subtyping and clonal relatedness of human and cattle verotoxin-producing Escherichia coli O157:H7 isolates. 61
32247643 2020
14
Biochemical analysis of patients with mutations in MTHFD1 and a diagnosis of methylenetetrahydrofolate dehydrogenase 1 deficiency. 61
32414565 2020
15
Rasburicase in hemolytic uremic syndrome related to Shiga toxin-producing Escherichia coli: a report of nine cases. 61
32152725 2020
16
Rasburicase improves the outcome of acute kidney injury from typical hemolytic uremic syndrome. 61
32561994 2020
17
Valid Presumption of Shiga Toxin-Mediated Damage of Developing Erythrocytes in EHEC-Associated Hemolytic Uremic Syndrome. 61
32512916 2020
18
Polyethylene Oxide (PEO) molecular size determines the severity of atypical thrombotic microangiopathy in a guinea pig model of acute intravenous exposure. 61
32579216 2020
19
Venom-Induced Consumption Coagulopathy Following Hump-Nosed Pit Viper (Genus: Hypnale) Envenoming in Sri Lanka: Uncertain Efficacy of Fresh Frozen Plasma. 61
32336579 2020
20
[Invasive pneumococcal disease and hemolytic uremic syndrome: new serotype]. 61
32470271 2020
21
The Role of Essential Oils against Pathogenic Escherichia coli in Food Products. 61
32570954 2020
22
Potentiation of Antibiotics by a Novel Antimicrobial Peptide against Shiga Toxin Producing E. coli O157:H7. 61
32572054 2020
23
Anti-Complement Factor H Autoantibody-Associated Atypical Hemolytic Uremic Syndrome. 61
31713210 2020
24
The familial risk of developing atypical Hemolytic Uremic Syndrome. 61
32488248 2020
25
Is ravulizumab the new treatment of choice for atypical hemolytic uremic syndrome (aHUS)? 61
32444092 2020
26
Late renal recovery after treatment over 1 year post-onset in an atypical hemolytic uremic syndrome: a case report. 61
32571244 2020
27
Mutation of complement factor B causing massive fluid-phase dysregulation of the alternative complement pathway can result in atypical hemolytic uremic syndrome. 61
32540405 2020
28
The long-acting C5 inhibitor, Ravulizumab, is effective and safe in adult patients with atypical hemolytic uremic syndrome naïve to complement inhibitor treatment. 61
32299680 2020
29
The limited knowledge of placental damage due to neglected infections: ongoing problems in Latin America. 61
32482148 2020
30
Distribution of Locus of Adhesion and Autoaggregation and hes Gene in STEC Strains from Countries of Latin America. 61
32504321 2020
31
Long-term outcomes and response to treatment in diacylglycerol kinase epsilon nephropathy. 61
32386968 2020
32
Eculizumab treatment of thrombotic microangiopathy in a patient with ulcerative colitis. 61
31612381 2020
33
Deficiency of CFHR plasma proteins and autoantibody positive hemolytic uremic syndrome: treatment rationale, outcomes, and monitoring. 61
32529325 2020
34
Discovery of 4-((2S,4S)-4-Ethoxy-1-((5-methoxy-7-methyl-1H-indol-4-yl)methyl)piperidin-2-yl)benzoic Acid (LNP023), a Factor B Inhibitor Specifically Designed To Be Applicable to Treating a Diverse Array of Complement Mediated Diseases. 61
32073845 2020
35
Effect of Rare Coding Variants in the CFI Gene on Factor I Expression Levels. 61
32510551 2020
36
Thomsen-Friedenreich antigen activation as a predictor for clinical outcome of pediatric patients with invasive pneumococcal disease. 61
32156480 2020
37
Interactions of Shiga toxin-producing Escherichia coli with leafy green vegetables. 61
32125677 2020
38
The Prevalence and Incidence of Hemolytic Uremic Syndrome in Iran, a Systematic Review and Meta-analysis. 61
32361693 2020
39
A nationwide cross-sectional analysis of thrombotic microangiopathy in the Japan Renal Biopsy Registry (J-RBR). 61
32415379 2020
40
Molecular Biology of Escherichia Coli Shiga Toxins' Effects on Mammalian Cells. 61
32456125 2020
41
Zebrafish embryo sensitivity test as in vivo platform to anti-Shiga toxin compound screening. 61
32449119 2020
42
Involvement of NF-κB1 and the Non-Canonical NF-κB Signaling Pathway in the Pathogenesis of Acute Kidney Injury in Shiga-Toxin-2-Induced Hemolytic-Uremic Syndrome in Mice. 61
32433206 2020
43
Ten-year outcome of Eculizumab in kidney transplant recipients with atypical hemolytic uremic syndrome- a single center experience. 61
32434487 2020
44
Renal Biopsy Prognostic Findings in Children With Atypical Hemolytic Uremic Syndrome. 61
32406813 2020
45
A case report of recurrent acute pancreatitis associated with life threatening atypical hemolytic uremic syndrome. 61
32481360 2020
46
Optic coherence tomography angiography follow-up in a case of Purtscher-like retinopathy due to atypical hemolytic uremic syndrome. 61
30841747 2020
47
Atypical Hemolytic Uremic Syndrome (p.Gly1110Ala) with Autoimmune Disease. 61
32361709 2020
48
European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) Complement Guideline: Deficiencies, Diagnosis, and Management. 61
32064578 2020
49
[The complement system-a "hot topic" not only for kidney diseases]. 61
32240352 2020
50
Complementopathies and precision medicine. 61
32310222 2020

Variations for Hemolytic-Uremic Syndrome

ClinVar genetic disease variations for Hemolytic-Uremic Syndrome:

6 ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 DGKE NM_003647.3(DGKE):c.888+40A>GSNV Pathogenic 548648 rs1555599211 17:54925466-54925466 17:56848105-56848105

Expression for Hemolytic-Uremic Syndrome

Search GEO for disease gene expression data for Hemolytic-Uremic Syndrome.

Pathways for Hemolytic-Uremic Syndrome

Pathways related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.7 VWF HP CFI CFHR3 CFH CFB
2
Show member pathways
11.96 CFI CFHR3 CFH CFB CD55 CD46
3
Show member pathways
11.72 CFI CFHR3 CFH CFB CD55 CD46
4 11.66 VWF THBD F3 CFI CFHR5 CFHR4
5 11.64 CFI CFH CFB C3 C2
6
Show member pathways
11.53 CFB CD55 C3 C2

GO Terms for Hemolytic-Uremic Syndrome

Cellular components related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 10.02 VWF HP CFI CFHR3 CFH CFB
2 extracellular space GO:0005615 9.93 THBD HP F3 CFI CFHR3 CFHR1
3 cell surface GO:0009986 9.8 THBD F3 CD55 CD46 CD40LG C3
4 extracellular region GO:0005576 9.53 VWF HP F3 CFI CFHR5 CFHR4
5 blood microparticle GO:0072562 9.43 HP CFHR3 CFHR1 CFH CFB C3

Biological processes related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.98 CFI CFH CFB CD55 CD46 C3
2 proteolysis GO:0006508 9.93 HP CFI CFB C2 ADAMTS13
3 immune system process GO:0002376 9.92 HP CFI CFH CFB CD55 CD46
4 blood coagulation GO:0007596 9.81 VWF THBD F3 ADAMTS13
5 complement activation, classical pathway GO:0006958 9.8 CFI CD55 CD46 C3 C2
6 platelet activation GO:0030168 9.76 VWF DGKE CD40LG ADAMTS13
7 negative regulation of protein binding GO:0032091 9.7 CFHR5 CFHR2 CFHR1
8 hemostasis GO:0007599 9.67 VWF THBD F3 ADAMTS13
9 complement activation GO:0006956 9.55 CFHR1 CFH CFB C3 C2
10 regulation of complement-dependent cytotoxicity GO:1903659 9.49 CFH CD55
11 positive regulation of apoptotic cell clearance GO:2000427 9.48 C3 C2
12 cytolysis by host of symbiont cells GO:0051838 9.43 CFHR5 CFHR2 CFHR1
13 regulation of complement activation GO:0030449 9.36 CFI CFHR5 CFHR4 CFHR2 CFHR1 CFH
14 complement activation, alternative pathway GO:0006957 9.26 CFHR5 CFH CFB C3

Molecular functions related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.91 VWF THBD MMACHC HP F3 CFI
2 serine-type endopeptidase activity GO:0004252 9.02 HP F3 CFI CFB C2

Sources for Hemolytic-Uremic Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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